Pediatric Idiopathic Intracranial Hypertension

Genevieve Mercille, MD* 2. Luis H. Ospina, MD† 1. *Resident in Ophthalmology, Pediatric and Neuro-Ophthalmology Sections, Ste-Justine Hospital, Montreal, Quebec, Canada 2. †Associate Professor in Ophthalmology, University of Montreal, Montreal, Quebec, Canada After completing this article, readers should be able to: 1. List the diagnostic criteria for idiopathic intracranial hypertension (IIH). 2. Discuss the epidemiology, risk factors, and clinical manifestations of IIH in a pediatric population. 3. Describe the differential diagnoses and conditions associated with IIH. 4. Suggest appropriate therapeutic options for IIH. 5. Identify the principal complication of IIH and how it may be prevented. Idiopathic intracranial hypertension (IIH), previously referred to as pseudotumor cerebri or benign intracranial hypertension, was recognized initially in adults by Quincke in 1893 as “meningitis serosa.” (1) The syndrome is characterized by elevated intracranial pressure (ICP) without any evident underlying neurologic disease. The modified Dandy criteria, which were developed based on an adult population, can assist in establishing the diagnosis of IIH (Table 1) (2). | | || * Adapted from Friedman and Jacobson (3). Table 1. Diagnostic Criteria for Idiopathic Intracranial Hypertension Interestingly, children who have IIH may display a greater spectrum of clinical presentations than adults, and the disorder may have special epidemiologic characteristics in children. IIH occurs most commonly in young adults and rarely is seen in those older than age 45 years. The overall annual incidence is 0.9 per 100,000 individuals, (4) and there is a strong female predilection among affected adults. The incidence of IIH increases to 3.5 per 100,000 in women ages 20 to …